Update on Antibiotics for Infection Control in Cystic Fibrosis

Stephen Kirkby; Kimberly Novak; Karen McCoy

Disclosures

Expert Rev Anti Infect Ther. 2009;7(8):967-980. 

In This Article

Abstract and Introduction

Abstract

Cystic fibrosis pulmonary disease is characterized by chronic and recurrent infection, airway inflammation, bronchiectasis and progressive obstructive lung physiology. Advances in the treatment of common airway pathogens such as Pseudomonas aeruginosa have led to a marked improvement in overall survival. However, antibiotic treatment options are often limited by multidrug resistance, potential toxicities and treatment burden to individual patients. While appropriate anti-infective therapy reduces bacterial density in the airways and may result in clinical improvement, true eradication of airway infection is seldom achieved except for early-stage infections. This review summarizes current approaches for acute and chronic anti-infective therapy in cystic fibrosis.

Introduction

Cystic fibrosis (CF) is the most common fatal genetic disease affecting Caucasian populations worldwide. This autosomal recessive disorder is caused by abnormalities in the cystic fibrosis transmembrane conductance regulator (CFTR) protein, which regulates chloride ion transport across cell membranes. Dysfunctional or insufficient levels of the CFTR protein are believed to result in the development of thick and viscous mucus in many organ systems. The respiratory tract is often the most severely affected system, and pulmonary disease is characterized by chronic and recurrent infection, inflammation and the development of bronchiectasis. Respiratory failure is the most common cause of death.[1]

Since its initial description in the late 1930s, there have been many significant advances in the management of CF. Current standard of care includes regular chest physiotherapy, maximization of nutritional status, careful screening for and treatment of comorbid conditions, and prompt and aggressive treatment of respiratory infections.[2,3] The result of this comprehensive management has been a consistent increase in overall survival; it is also associated with increasing burden of care. The most recent patient registry data from the US Cystic Fibrosis Foundation (CFF) shows a median life expectancy approaching 38 years of age (Figure 1).[4]

Figure 1.

Improved survival in cystic fibrosis.

Anti-infective therapy has evolved over the past 70 years to include formulations of intravenous, oral and inhaled medications. These therapies are tailored to individual patients based on several clinical factors, including the presence of specific chronic airway pathogens, severity of acute exacerbation and underlying chronic lung disease, and need for inpatient versus outpatient treatment. There are also several anti-infective strategies for the chronic maintenance of lung health. A comprehensive review of the pathophysiology and management of infections in CF has been published.[3] Furthermore, recent US and UK guidelines are available, which outline current standards of antibiotic therapy.[5,6] The purpose of this article is to review recent knowledge on the antibiotic treatment of pulmonary infections in CF, with particular emphasis on drugs used for the treatment of clinically important and emerging pathogens.

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